AIM: Acquired hepatocerebral degeneration (AHD) is an exceptional type of hepatic encephalopathies (HE). It is characterized by neuropsychiatric and extrapyramidal symptomathology similar to that seen in hepatolenticu...AIM: Acquired hepatocerebral degeneration (AHD) is an exceptional type of hepatic encephalopathies (HE). It is characterized by neuropsychiatric and extrapyramidal symptomathology similar to that seen in hepatolenticular degeneration (Wilson's disease). In this paper, we report a case of AHD with unusual presenting features.METHODS: A 28-year-old man with AHD was described and the literature was reviewed.RESULTS: The man had a history of HBV-related liver cirrhosis. He was admitted to our hospital with apathy,dysarthria, mild consciousness impairment and extrapyramidal symptoms after hematemesis. By review of the literature,cases with AHD often did not present consciousness impairment. So our case was once diagnosed incorrectly as Wilson's disease.CONCLUSION: AHD is a rare syndrome and its variable clinical manifestations make it difficult to be diagnosed.But we believe that extensive examination and thorough understanding of the disease are beneficial to a correct diagnosis. Moreover, biocoene is effective in treating the case.展开更多
BACKGROUND Metastatic small bowel low-grade neuroendocrine tumors(NETs)have a good prognosis.Surgery is the only curative treatment;however,this may induce advanced liver disease,particularly in long-term survivor pat...BACKGROUND Metastatic small bowel low-grade neuroendocrine tumors(NETs)have a good prognosis.Surgery is the only curative treatment;however,this may induce advanced liver disease,particularly in long-term survivor patients.Acquired hepatocerebral degeneration or Parkinsonism in cirrhosis is characterized by rapidly progressive extrapyramidal symptoms in patients with advanced liver disease.CASE SUMMARY A 70-year-old man presented to the emergency department with diminished consciousness and disorientation,and was diagnosed with hepatic encephalopathy.The patient was diagnosed in 1993 with a metastatic small bowel NET,for which he twice underwent hepatic surgery,with metastatic resection in 1993 and a right hepatectomy in 2002 to remove two hepatic metastases.In 2003,the patient started first-line chemotherapy and in 2004 started the first of three consecutive biological treatments,followed by radio-molecular therapy,achieving stable disease for 14 years.Disease progression was identified and he underwent an endoscopic retrograde cholangiopancreatography.However,in 2019 advanced liver disease was identified.We diagnosed the development of acquired hepatocerebral degeneration,an unusual long-term side effect after multiple hepatic procedures.CONCLUSION The importance of regular and ongoing surveillance in long-term NET survivors who undergo hepatic procedures should be integrated into the therapeutic management plan,as some of these negative outcomes could be prevented.展开更多
文摘AIM: Acquired hepatocerebral degeneration (AHD) is an exceptional type of hepatic encephalopathies (HE). It is characterized by neuropsychiatric and extrapyramidal symptomathology similar to that seen in hepatolenticular degeneration (Wilson's disease). In this paper, we report a case of AHD with unusual presenting features.METHODS: A 28-year-old man with AHD was described and the literature was reviewed.RESULTS: The man had a history of HBV-related liver cirrhosis. He was admitted to our hospital with apathy,dysarthria, mild consciousness impairment and extrapyramidal symptoms after hematemesis. By review of the literature,cases with AHD often did not present consciousness impairment. So our case was once diagnosed incorrectly as Wilson's disease.CONCLUSION: AHD is a rare syndrome and its variable clinical manifestations make it difficult to be diagnosed.But we believe that extensive examination and thorough understanding of the disease are beneficial to a correct diagnosis. Moreover, biocoene is effective in treating the case.
文摘BACKGROUND Metastatic small bowel low-grade neuroendocrine tumors(NETs)have a good prognosis.Surgery is the only curative treatment;however,this may induce advanced liver disease,particularly in long-term survivor patients.Acquired hepatocerebral degeneration or Parkinsonism in cirrhosis is characterized by rapidly progressive extrapyramidal symptoms in patients with advanced liver disease.CASE SUMMARY A 70-year-old man presented to the emergency department with diminished consciousness and disorientation,and was diagnosed with hepatic encephalopathy.The patient was diagnosed in 1993 with a metastatic small bowel NET,for which he twice underwent hepatic surgery,with metastatic resection in 1993 and a right hepatectomy in 2002 to remove two hepatic metastases.In 2003,the patient started first-line chemotherapy and in 2004 started the first of three consecutive biological treatments,followed by radio-molecular therapy,achieving stable disease for 14 years.Disease progression was identified and he underwent an endoscopic retrograde cholangiopancreatography.However,in 2019 advanced liver disease was identified.We diagnosed the development of acquired hepatocerebral degeneration,an unusual long-term side effect after multiple hepatic procedures.CONCLUSION The importance of regular and ongoing surveillance in long-term NET survivors who undergo hepatic procedures should be integrated into the therapeutic management plan,as some of these negative outcomes could be prevented.
